Pediatric Nephrology

, Volume 20, Issue 8, pp 1098–1105 | Cite as

Childhood thin GBM disease: review of 22 children with family studies and long-term follow-up

  • Carla Carasi
  • William G. Van’t Hoff
  • Lesley Rees
  • R. Anthony Risdon
  • Richard S. Trompeter
  • Michael J. Dillon
Original Article


Thin glomerular basement membrane (GBM) disease is generally known to have a good renal prognosis, although renal insufficiency has sometimes been reported and the overlap with Alport syndrome implies that a good prognosis cannot be guaranteed. In order to shed light on long-term prognosis of thin GBM disease we have retrospectively evaluated 22 children with persistent haematuria and biopsy-proven thin GBM. Mean follow up was 7 years (range 2–17 years), mean age at onset was 7 years (range 1.5–15). Biopsies were performed a mean of 3.8 years after detection of hematuria. The light microscopy (LM) and immunofluorescence (IF) findings were essentially unremarkable in all of the children, while electron microscopy (EM) showed thinning of the GBM in all cases and no changes characteristic of Alport syndrome. The family history was positive for renal disease in 17 (77.3%) patients with hematuria in 8 (36.3%) families, and hematuria with renal failure (RF) or deafness in 9 (40.9%). It was completely negative for renal disease in 4 (18.2%) and unavailable in 1 (4.5%). Four patients (18%) showed a decline in renal function after 6, 8, 9 and 12 years of follow-up, and 1 of these also developed hearing impairment. None developed hypertension.

Our study suggests that thin GBM disease is not always benign and a child with thin GBM should never be assigned such a prognosis, especially if there is a family history of renal impairment or deafness, where careful follow-up is needed due to the risk of late onset renal failure.


Thin glomerular basement membrane disease Familial benign hematuria Alport syndrome 



We acknowledge the support of Dr Frances Flinter, Clinical Director of Children’s Service & Genetics & Consultant Clinical Geneticist at Guy’s & St. Thomas’ Hospital, London, who kindly undertook the genetic analysis. We also acknowledge Professor Graziella Zacchello, Dr. Luisa Murer, Dr. Giovanni Montini (Pediatric Nephrology Consultants), and Dr Lina Artifoni (Consultant Geneticist) from the Department of Pediatrics, Dialysis and Transplant Unit, University of Padova, Italy, for kindly reviewing the manuscript and for their helpful comments. The results of this study were presented in part at the World Congress of Nephrology at Berlin, in 2003.


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Copyright information

© IPNA 2005

Authors and Affiliations

  • Carla Carasi
    • 3
  • William G. Van’t Hoff
    • 1
  • Lesley Rees
    • 1
  • R. Anthony Risdon
    • 2
  • Richard S. Trompeter
    • 1
  • Michael J. Dillon
    • 1
  1. 1.Department of NephrologyGreat Ormond Street Hospital for Children NHS TrustLondonUK
  2. 2.Department of HistopathologyGreat Ormond Street Hospital for Children NHS TrustUK
  3. 3.Department of Paediatrics, Dialysis and Transplant UnitUniversity of PadovaPadovaItaly

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